NCT04095351

Connective Tissue Diseases and Lung Manifestations

Connective Tissue Diseases and Lung Manifestations Prospective Trial With Focus on Systemic Sclerosis (Colipris)

• Status: Recruiting
• Phase: —
• Study type: Observational
• Enrollment: 120 (estimated)
• Sponsor: Medical University Innsbruck · Academic / Other
• Sex: All
• Age: 18 Years – 80 Years
• Healthy volunteers: Not accepted

Summary

Despite a number of prospective studies already initiated in the past years, the current epidemiology and course of interstitial lung disease (ILD) and pulmonary hypertension (PH) in patients with connective tissue disease (CTD) is still not well defined, particularly regarding its prevalence, incidence and the management of a broad spectrum of disease presentations. Major challenges include the identification of patients with progressive disease, the appropriate time point of therapeutic intervention and the underlying driver of disease (inflammatory or pro-fibrotic stimulus or both?). To address these issues in Western Austria, a progressive registry of patients with CTD exploring routine clinical and pathophysiological characteristics of ILD and PH will be conducted. This multidisciplinary, prospective and observational registry aims to collect comprehensive clinical data on incidence, prevalence and course of disease regarding all PH and ILD presentations in a real-world setting.

Detailed description

Specifically, this registry will collect demographic data, disease-related clinical data, routine laboratory values (including antibody-profile and iron status), diagnostic procedures, significant comorbidities, therapeutic managements (e.g. thoracic ultrasound, HRCT), and disease outcomes over 10 years. In addition, blood biobank samples for translational research will be collected in a subgroup of patients with systemic sclerosis over serial time points to study the systemic inflammatory and profibrotic phenotype of patients. In summary, this registry will monitor the disease course of pulmonary manifestations of patients with CTD and may be hypothesis-generating and provide new insights in underlying inflammatory/pro-fibrotic patterns.

Conditions

• Connective Tissue Diseases
• Interstitial Lung Disease
• Systemic Sclerosis

Interventions

Type	Name	Description
DIAGNOSTIC_TEST	Pulmonary function test	Spirometry or plethysmography, measurement of diffusion capacity
DIAGNOSTIC_TEST	Imaging	Thoracic ultrasound, HRCT as scheduled within routine clinical examinations, echocardiography
BIOLOGICAL	Blood sampling	Standard laboratory test for CTDs as part of routine clinical examination

Timeline

• Start date: 2019-12-09
• Primary completion: 2021-03-01
• Completion: 2034-10-01
• First posted: 2019-09-19
• Last updated: 2020-05-27

Locations

1 site across 1 country: Austria